Hereditary anhidrotic ectodermal dysplasia is a rare condition characterized by underdeveloped ectodermal structure including the skin, teeth or skin appendages. The patient has characteristic feature of anhidrosis, hypotrichosis and defective dentition. We experienced a case of hereditary anhidrotic ectodermal dysplasia in a l-month-old male infant who had unexplained recurring fever, anhidrosis and characteristic facial feature, so we established the diagnosis with clinical feature and skin biopsy.
Biopsy ; Dentition ; Diagnosis ; Ectoderm ; Ectodermal Dysplasia* ; Fever ; Humans ; Hypohidrosis ; Hypotrichosis ; Infant ; Male ; Skin ; Tooth

Goltz syndrome is known as a rare mesoectodermal hereditary disease, characterized by focal dermal atrophies with hernias of adipose tissue and also associated with a multitude of possible skeletal, dental, ophthalmological and other abnormalities. We experienced a case of Goltz syndrome. An one day old female newborn had focal atrophic and telangiectatic skin lesions, microphthalmia, syndactyly and urinary tract abnormality. The finding of skin biopsy was consistent with focal dermal hypoplasia. We report the case with a brief review and related literatures.
Adipose Tissue ; Atrophy ; Biopsy ; Female ; Focal Dermal Hypoplasia* ; Genetic Diseases, Inborn ; Hernia ; Humans ; Infant, Newborn ; Microphthalmos ; Skin ; Syndactyly ; Urinary Tract

We report a case of subcutaneous phaeohyphomycosis caused by Exophiala(E,) jeanselmei in a 66-year-old female, who showed a mild tender, 4.5x3.5cm sized, erythematous cystic mass with satellite lesions on the left forearm for 4 months. Histopathologically, suppurative granulomatous inflammation, brownish conidia in a chain and hyphae were observed. Fungal culture grew out the typical black-gray velvety colonies of E. jeanselmei after 2 weeks. The isolate grow well at 25 C, but very poorly at 37 C. No growth could be observed at 40 C. Sporulation adequate for evaluation was present on the malt extract agar. We confirmed E. jeanselmei by colony and microscopic morphology, temperature tolerance and sugar assimilation tests. The patient had been treated with itraconazole for 6 momths. Complete remission was observed.
Agar ; Aged ; Exophiala* ; Female ; Forearm ; Humans ; Hyphae ; Inflammation ; Itraconazole ; Phaeohyphomycosis* ; Spores, Fungal

OBJECTIVE: To describe the videofluoroscopic findings of quadriplegic patients with dysphagia and to assess the predisposing factors of dysphagia in quadriplegic patients. METHOD: Six quadriplegic patients with a dysphagia within 3 months from the injury were included in this study and videofluoroscopic evaluations for both lateral and AP views were performed and evaluated. RESULT: A variety of swallowing deficits that involve both oral and pharyngeal phases were detected in the videofluoroscopic study. The presumed predisposing factors of dysphagia were a surgical stabilization of cervial vertebrae, inadequate neck extension due to cervical orthosis, history of mechanical ventilation, cervical traction, tracheostomy state and cranial nerve injury. CONCLUSION: When there are symptoms that suggest a swallowing problem such as a frequent reflex coughing and voice change in quadriplegic patients, videofluoroscopic study will help to prevent the pulmonary complications and to determine the successful swallowing strategies.
Causality ; Cough ; Cranial Nerve Injuries ; Deglutition ; Deglutition Disorders* ; Humans ; Neck ; Orthotic Devices ; Quadriplegia ; Reflex ; Respiration, Artificial ; Spinal Cord Injuries ; Spine ; Tracheostomy ; Traction ; Voice

BACKGROUND AND OBJECTIVES: There are two types of methods in the underlay technique that are employed according to the graft insertion site. The purpose of this study is to evaluate the short term follow-up results of air-bone conduction threshold changes according to the insertion of temporalis muscle fascia to medial and lateral side of malleus in type I tympanoplsty with simple mastoidectomy. MATERIALS AND METHOD: 72 patients with perforated tympanic membrane over the half size in pars tensa were operated in type I tympanoplasty with simple mastoidectomy. Patients were divided into two groups according to the insertion of fascia of temporalis muscle to either i) lateral side of the malleus (group A, 32 cases) or ii) medial side of the malleus (group B, 40 cases). Each group was divided into three subgroups according to size of perforation in the tympanic membranes, ranging from moderate, large, to total. Difference in the air-borne conduction threshold changes between the first preoperative and postoperative year was analyzed for each group. RESULTS: The differences in the pre and postoperative air-bone conduction gap (ABG) between the group A and B were 14.4+/-7.1 dB, 10.4+/-6.2 dB, respectively. There was no statistical difference between the two groups (p>0.05). The pre and postoperative ABG differences for different sizes of perforation in the tympanic membrane in group A were 17.5+/-12.2 dB (moderate), 14.1+/-7.4 dB (large), 14.5+/-8.5 dB (total), and in group B were 9.5+/-4.8, dB (moderate), and 9.3+/-7.2 dB (large), and 12.0+/-7.1 dB (total). There was no statistical difference between the subgroups of the same group (p>0.05). The pre and postoperative bone conduction threshold of group A decreased at 250, 500, 1000, and 2000 Hz but increased at 3000, 4000 Hz. The threshold of group B decreased at all frequency. Postoperative reperforations were observed in just 2 cases of the group B. One case was observed at 5 postoperative months in the large perforation group, and the other case was observed at 15 months in the total perforation group, with the former case healing spontaneously at 6 months. CONCLUSION: The results suggest that the two methods of underlay technique could be selected properly by location of perforation, middle ear anatomy and its pathological state, and continuity of ossicles, as well as per operator's preference. When the perforation is located at the anterior, anterosuperior and anteroposterior portion of tympanic membrane, the tympanic membrane is thickened severely so the boundary becomes uncertain between the middlear mucosa and the tympanic membrane. The insertion of fascia to lateral side of malleus is therefore recommended.
Bone Conduction ; Ear, Middle ; Fascia* ; Follow-Up Studies* ; Hearing* ; Humans ; Malleus ; Mucous Membrane ; Transplants ; Tympanic Membrane ; Tympanoplasty*

PURPOSE: To determine the clinical and pathological risk factors for subsequent bladder tumor in patients with primary upper tract urothelial tumor, we retrospectively analyzed patients with upper tract urothelial tumor, focusing on the clinicopathological features of subsequent bladder tumor. MATERIALS AND METHODS: Risk factors, disease free rate and survival were assessed with clinicopathological features in 56 patients with upper tract urothelial tumor operated between 1989 and 1998. We excluded the patients with lymph node metastasis or distant metastasis, those with a short period of follow-up, and those having a previous history of bladder tumor. Risk factors such as sex, age, location of tumor, size of tumor, number of tumor, synchronous bladder tumor, preoperative urine cytology, stage, grade, operation method, and adjuvant chemotherapy were investigated. RESULTS: Initial subsequent bladder tumor was found in 36 patients (53.6%) during follow-up period of 44 months (range 13 to 111). Among several clinicopathological factors examined, only urine cytology was significantly correlated with the incidence of subsequent bladder tumor (p<0.05). Sex, age, location of tumor, size of tumor, number of tumor, synchronous bladder tumor, stage, grade, operation method, or adjuvant chemotherapy did not affect subsequent bladder tumor recurrence. There was no significant difference in survival rates between the patients with and without subsequent bladder tumor. CONCLUSIONS: Of the clinical and pathological risk factors for subsequent bladder tumor in patients with primary upper tract urothelial tumor, only preoperative urine cytology was significantly correlated with subsequent bladder tumor.
Chemotherapy, Adjuvant ; Follow-Up Studies ; Humans ; Incidence ; Lymph Nodes ; Neoplasm Metastasis ; Recurrence ; Retrospective Studies ; Risk Factors* ; Survival Rate ; Urinary Bladder Neoplasms* ; Urinary Bladder*

Obesity occurs when a person's calorie intake exceeds the amount of energy burns, which may lead to pathologic growth of adipocytes and the accumulation of fat in the tissues. In this study, the effect and mechanism of pear pomace extracts on 3T3-L1 adipocyte differentiation and apoptosis of mature adipocytes were investigated. The effects of pear pomace extract on cell viability and the anti-adipogenic and proapoptotic effects were investigated via MTT assay, Oil red O staining, western blot analysis and apoptosis assay. 3T3-L1 preadipocytes were stimulated with DMEM containing 10% FBS, 0.5 mM 3-isobutyl-1-methylxanthine (IBMX), 5 microg/ml insulin and 1 microM dexamethasone for differentiation to adipocytes. 3T3-L1 cells were cultured with PBS or water extract of pear pomace. Water extract of pear pomace effectively inhibited lipid accumulations and expressions of PPAR-gamma and C/EBPalpha in 3T3-L1 cells. It also increased expression of p-AMPK and decreased the expression of SREBP-1c and FAS in 3T3-L1 cells. The induction of apoptosis was observed in 3T3-L1 cells treated with pear pomace. These results indicate that pear pomace water extract inhibits adipogenesis and induces apoptosis of adipocytes and thus can be used as a potential therapeutic substance as part of prevention or treatment strategy for obesity.
1-Methyl-3-isobutylxanthine ; 3T3-L1 Cells ; Adipocytes* ; Adipogenesis* ; Apoptosis* ; Blotting, Western ; Burns ; Cell Survival ; Dexamethasone ; Insulin ; Obesity ; Pyrus* ; Sterol Regulatory Element Binding Protein 1 ; Water*

BACKGROUND/OBJECTIVES: In this study, the inhibitory effect of Erythronium japonicum extracts on the metastasis of MDA-MB-231 human breast cancer cell line was determined. MATERIALS/METHODS: Cells were cultured with DMSO or with 50, 75, 100 or 250 microg/ml of Erythronium japonicum methanol or ethanol extract. RESULTS: Both methanol and ethanol extracts significantly inhibited the growth and induced apoptosis of MDA-MB-231 cells in a dose-dependent manner. Erythronium japonicum extracts inhibited the adhesion of MDA-MB-231 cells. The invasion of breast cancer cells was suppressed by Erythronium japonicum extracts in a dose-dependent manner. The motility and MMP-2 and MMP-9 activities were also inhibited by both methanol and ethanol extracts. CONCLUSIONS: Our results collectively indicate that Erythronium japonicum extracts inhibit the growth, adhesion, migration and invasion as well as induce the apoptosis of human breast cancer cells. Clinical application of Erythronium japonicum as a potent chemopreventive agent may be helpful in limiting breast cancer invasion and metastasis.
Apoptosis ; Breast Neoplasms* ; Cell Line ; Dimethyl Sulfoxide ; Ethanol ; Humans ; Methanol ; Neoplasm Metastasis*

We found an error in our published article. Figure 1 should be corrected.

No abstract available.
Adult* ; Communicable Diseases* ; Humans ; Immunization*